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Clear cell sarcoma of the kidney is a rare variety of renal tumor accounting for less than 5% of all pediatric renal tumors. Cardiopulmonary bypass along with hypothermic circulatory arrest is frequently used for management of tumor thrombus extending into supra-hepatic inferior vena cava and right atrium. In this paper, we present a strategy of avoiding circulatory arrest and hypothermia and thereby fast-tracking the recovery in managing a case of clear cell sarcoma of the kidney in a 3.5-year-old child with tumor thrombus extending into the right atrium.

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OBJECTIVE: To assess the feasibility of initiating enteral nutrition support with first 24 h of congenital heart repairs in neonates and Infants and its impact on outcomes following surgery. DESIGN: It is a prospective randomized control single blind study. SETTING: It is a single centre prospective study carried out in a tertiary care centre at Pediatric cardiac intensive care unit. PATIENTS: All patients with the cyanotic congenital heart disease with increase pulmonary blood flow, weighing less than 5 kg and undergoing congenital heart repair during the study period were included in the study. Patients with single ventricle status, those undergoing palliative procedures (PA band), open chest, requiring ECMO before leaving operating room, having any other contraindication for starting enteral feeding or those who refuse for consent were excluded from study. INTERVENTIONS: The patients were randomized into two groups. Group 1 received trophic feeds (10-20 ml/kg/day) starting 4-6 h after surgery while feeds children in group 2 were kept NPO and received feeds after 48 h after surgery. MEASUREMENTS AND MAIN RESULTS: 15 children enrolled in both the groups. Both pre-operative and intraoperative variables were comparable in both the groups. There was no complication (vomiting, diarrhea, NEC, bowel necrosis) noted in the children who received feeds after surgery. Mean duration of mechanical ventilation in the feeds group was 58.2 ± 4.71 h, which was less then significantly less than those in the NPO group (P value 0.05). Similarly, duration of ICU stay was only 179.04 ± 41.28 h in feeds group as compared to 228.72 ± 85.44 h in the NPO group. CONCLUSIONS: Neonates and Infants tolerate feeds immediately following congenital heart repairs. Moreover, feeds appear to decrease duration of mechanical ventilation and duration of ICU stay.

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Repair of congenital heart disease in the presence of high pulmonary pressure has always been a contentious issue. Pulmonary vascular resistance (PVR) is considered important for establishing operability in these patients. However, PVR estimation is not always accurate and cannot solely be relied upon to make critical decision of operability. Clinical examination, chest X-ray, and echocardiography are also important indicators of pulmonary vascular disease. Knowledge of pits and falls of each investigation is important for appropriate management in these patients. We present a case report of successfully operated, 6-year-old child with anomalous origin of the right pulmonary artery from aorta, deemed inoperable on the basis of PVR estimation.

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Parachute valve is the malformation of an atrioventricular valve in which the tension apparatus springs from a single papillary muscle or group of muscles. Parachute tricuspid valve is a rare anomaly with no surgically repaired case to date. We describe a case of parachute deformity of the tricuspid valve leading to hemodynamically significant severe tricuspid stenosis. The present case was successfully repaired surgically along with atrial septal defect (ASD) and ventricular septal defect (VSD) closure.

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CONTEXT: Levosimendan is a new generation inotrope with calcium sensitizing properties and proven benefits in adults. AIMS: This study investigates the use of levosimendan as a first line inotrope in congenital heart surgery. SETTINGS AND DESIGN: Prospective, observational study in a tertiary care center. MATERIALS AND METHODS: One hundred and ten patients undergoing congenital cardiac surgery received levosimendan at a loading dose of 12 mcg/kg during rewarming on cardiopulmonary bypass followed by continuous infusion of 0.1 mcg/kg/min for 48 h. Hemodynamic parameters were recorded at the time of admission to Intensive Care Unit, and at 3 h, 6 h, 12 h, 24 h, and 48 h thereafter. STATISTICAL ANALYSIS: Categorical variables were compared using Chi-square test. Non-normally distributed quantitative variables were compared between groups using Kruskal-Wallis test. RESULTS: At discharge from operating room (OR), 36 (32.7%) patients required levosimendan alone to maintain optimum cardiac output, 59 (53.6%) patients required the addition of low-dose adrenaline (<0.1 mcg/kg/min) and 15 (13.6%) patients required either increment in adrenaline to high-dose (≥0.1 mcg/kg/min) or starting another inotrope/vasoactive agent. Overall, there were five mortalities. Hypotension leading to discontinuation of levosimendan was not found in any patient. Arrhythmias were observed in three patients. Fifty-four patients were extubated in the OR. CONCLUSIONS: Levosimendan-based inotropic regime offers optimized cardiac output with a well-controlled heart rate and a low incidence of arrhythmias in patients undergoing all categories of congenital heart surgeries.

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Coronary artery aneurysm (CAA) is defined as dilatation of a coronary artery segment to a diameter of more than 1.5-fold normal size. Rupture of CAA is a catastrophic event and may result in sudden death or myocardial infarction. We report this unusual case of contained rupture of the left circumflex CAA.

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